Treatment of Acquired von Willebrand Disease due to Extracorporeal Membrane Oxygenation in a Pediatric COVID-19 Patient with Vonicog Alfa: A Case Report and Literature Review

Abstract

Acquired von Willebrand disease (aVWD) is frequently observed in patients with the need for extracorporeal membrane oxygenation (ECMO). aVWD can be treated by plasma-derived concentrates containing factor VIII (FVIII) and/or von Willebrand factor (VWF) and recombinant VWF concentrate as well as adjuvant therapies such as tranexamic acid and desmopressin. However, all of these therapeutic options possibly cause thromboembolism. Therefore, the optimal treatment remains uncertain. This report presents a case of a 16-year-old patient suffering from severe acute respiratory distress syndrome due to coronavirus disease 2019 with the need of ECMO support. Our patient developed aVWD under ECMO therapy characterized by loss of high-molecular-weight multimers (HMWM) and severe bleeding symptoms following endoscopic papillotomy due to sclerosing cholangitis. At the same time standard laboratory parameters showed hypercoagulability with increased fibrinogen level and platelet count. The patient was successfully treated with recombinant VWF concentrate (rVWF; vonicog alfa; Veyvondi) combined with topic tranexamic acid application and cortisone therapy. rVWF concentrate vonicog alfa is characterized by ultra-large multimers and absence of FVIII. Patient could be successfully weaned from ECMO support after 72 days. Multimer analysis 1 week after ECMO decannulation showed an adequate reappearance of HMWM.

Keywords: acquired coagulation disorders; extracorporal circulation; von Willebrand disease.

Fig. 1

Presentation of patient's time course of disease since hospital admission (day 0). ICU, intensive care unit; NIV, noninvasive ventilation; HFNC, high-flow nasal cannula; PCR, polymerase chain reaction; BAL, bronchial lavage sampling; vv-ECMO, veno-venous extracorporeal membrane oxygenation; RBC, red blood cell transfusion; ERCP, endoscopic retrograde cholangiopancreatography; NAVA, neurally adjusted ventilator assist; pSO ₂ , partial oxygen saturation.

Fig 2

von Willebrand factor (VWF) multimer distributions on day 3 after bleeding occurred under and after ECMO therapy compared to healthy control. ECMO, extracorporeal membrane oxygenation; HMWMM, high molecular weight multimers.